Department of Neuro-Oncology/Neurosurgery, Saitama Medical University International Medical Center, 1397-1, Yamane, Hidaka, Saitama, 350-1298, Japan.
Department of Pathology, Saitama Medical University, Saitama, Japan.
Brain Tumor Pathol. 2021 Jul;38(3):243-249. doi: 10.1007/s10014-021-00401-6. Epub 2021 Apr 28.
Astroblastoma is an extremely rare brain tumor that has recently attracted attention owing to its association with MN1 gene alteration. However, its long-term clinical course remains unclear. We report a late recurrence of MN1-altered astroblastoma with unique pathological findings. A 24-year-old woman presented with seizures due to a left frontal lobe tumor. Gross total resection (GTR) was achieved, and the diagnosis was MN1-altered astroblastoma, which presented cell wrapping, i.e., presence of tumor cells enveloping one another. She received local radiotherapy (50 Gy). However, the tumor recurred after 12 years, and its size increased rapidly. The second surgery achieved GTR and confirmed increasing anaplasia. The patient was tumor-free for 1 year without any neurological deficits. This case implies the importance of long-term follow-up of MN1-altered astroblastoma. The pathological significance of cell wrapping in this case is unclear, but it may be associated with MN1-altered astroblastoma and should be noted in future cases.
星形母细胞瘤是一种极其罕见的脑肿瘤,最近由于其与 MN1 基因突变的关联而引起关注。然而,其长期临床病程尚不清楚。我们报告了一例 MN1 改变的星形母细胞瘤的晚期复发,具有独特的病理发现。一名 24 岁女性因左额叶肿瘤出现癫痫发作。实现了全切除(GTR),诊断为 MN1 改变的星形母细胞瘤,存在细胞包裹,即存在彼此包裹的肿瘤细胞。她接受了局部放疗(50Gy)。然而,肿瘤在 12 年后复发,且其大小迅速增大。第二次手术实现了 GTR,并证实了间变程度增加。患者在无任何神经功能缺损的情况下无肿瘤 1 年。该病例提示对 MN1 改变的星形母细胞瘤进行长期随访的重要性。该病例中细胞包裹的病理意义尚不清楚,但它可能与 MN1 改变的星形母细胞瘤有关,在未来的病例中应注意。